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Heme/Onc > RBC Disorders > Flashcards

RBC Disorders Flashcards

1
Q

Thalassemia Clinical Features

A 
minor= asymptomatic
major= sick

Anemia with normal iron stores

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2
Q

Thalassemia Diagnostics

A
  • GOLD STANDARD= hemoglobin electrophoresis
  • microcytic
  • ELEVATED Indirect bilirubin LDH
  • DECREASED Hbg (3-6)
  • normal/increased iron
  • normal TIBC
  • Ferritin normal/increase
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3
Q

Beta-Thalassemia Clinical Features

A
  • sxs begin 4-6months of age when switch from HgbF
  • retardation
  • abnormal facial structure
  • osteopenia
  • pathological fractures
  • jaundice
  • hepatosplenomegaly
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4
Q

Thalassemia Treatment

A
  1. blood transfusions w/iron chelator (deferoxamine)
  2. bone marrow transplant, splenectomy
  3. genetic counseling
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5
Q

Iron-Deficiency Anemia Characteristics

A
  1. In adults: SLOW BLEEDING: GI occult bleed secondary to PUD, NSAID, heavy menstrual blood loss
  2. Low dietary intake of iron in children, impoverished, pregnant
  3. gastronectomy (decreased absorption)
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6
Q

Iron-Deficiency Diagnostics

A
  1. Decreased H&H
  2. Smear: hypochromic, microcytic RBC
  3. DECREASED ferritin (under 20)
  4. DECREASED iron
  5. ELEVATED TIBC
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7
Q

Iron-Deficiency Treatment

A
  1. Ferrous Sulfate 325mg tid (titrate up)
    - anemia will be fixed in 2 months, but continue therapy for at least 6 months to replenish stores
    - iron supplementation during pregnancy and lactation is ESSENTIAL
  2. work-up for occult blood loss
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8
Q

Anemia of Chronic Disease Characteristics

A
  1. chronic inflammation (blocks iron absorption from the gut and the release of iron from the bone marrow)
  2. RA, SLE
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9
Q

Anemia of Chronic Disease Labs

A
  1. 70% normocytic, 30% microcytic
  2. elevated CRP
  3. microcytic
  4. opposite results of Fe-deficiency (other than Fe):
    - DECREASED iron
    - INCREASED ferritin
    - DECREASED TIBC
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10
Q

Anemia of Chronic Disease Treatment

A
  1. treat underlying cause!
  2. transfusions while symptomatic
  3. EPO and iron DO NOT HELP
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11
Q

Sideroblastic Anemia Characteristics

A
  1. reduced hemoglobin synthesis, causing build up of uron in mitochondria= RINGED-CELLS (sideroblasts)
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12
Q

Sideroblastic Anemia Causes

A
  1. lead poisoning
  2. alcoholism
  3. decreased copper
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13
Q

Sideroblastic Anemia DX

A
  1. GOLD STANDARD= bone marrow biopsy
  2. Increased Fe
  3. ferritin, TIBC wnl
  4. basophilic stippling of red blood cells
  5. microcytic
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14
Q

Sickle Cell Anemia Patho

A

-autosomal recessive Hgb SS
-sickling only occurs when homozygous
-sickling occurs due to acid, hypoxia, dehydration, temperatures
(AVOID high altitudes, deep sea diving)

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15
Q

Sickle Cell Clinical Features

A
  1. sxs occur at 6months yo (when Hgb F stops)
  2. vascular occlusions (ischemia causes pain)
  3. strokes
  4. chest syndrome
  5. avascular necrosis
  6. splenic sequestration
  7. delayed growth/puberty
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16
Q

Sickle Cell Diagnostic Studies

A
  1. hemoglobin electrophoresis
  2. during crisis: smear shows sickled cells
  3. ELEVATED retic count, indirect blilrubin, LDH
  4. ELEVATED WBC
17
Q

Sickle Cell Treatment

A
  1. hydroxyurea for SS patients (increases Hgb F)
  2. Symptomatic during pain crisis: analgesics, hypotonic fluids, rest
  3. stroke, sequestration, acute chest syndrome may require transfusion/exchange transfusion
18
Q

G6PD Deficiency Patho

A

decreased G6PD means RBC can’t protect against oxidative stress– POPS RBC

19
Q

G6PD Deficiency Oxidative Stresses

A

Cause episodic hemolysis:

  1. Fava beans
  2. dapsone, atovoquone (African prophylaxis)
  3. infection
  4. quinidine, sulfanomides
20
Q

G6PD Deficiency DX

A
  1. G6PD 6-8 weeks AFTER event (decrease)
  2. during episodes:
    - ELEVATED retics and indirect bilirubin
    - smear: bite cells, heinz bodies (denatured hemoglobin)
21
Q

G6PD Deficiency Treatment

A
  1. Hemolytic episodes are self-limited and RBC are replaced as soon as offending agent is stopped
  2. avoid oxidative drugs and fava beans
22
Q

Folic Acid Deficiency Characteristics

A
  1. usually from poor dietary intake (few WEEKS to deplete stores)
  2. folic acid is found in green-leafy vegetables
  3. ALCOHOLICS, anorexia, diet low in fruits/veggies
  4. pregnancy, chronic hemolytic anemia and exfoliative skin may need supplementation
23
Q

Folic Acid Clinical Features

A
  1. sore tongue (glossitis)
  2. GI sxs
  3. NO neuro sxs
24
Q

Folic Acid DX

A
  1. macro-ovalocytes and hypersegmented polymorphonuclear cells
  2. folate level under 150
  3. NORMAL MMA, elevated homocysteine
25
Q

Folic Acid Treatment

A
  1. Oral replacement (1mg/day)

2. avoid alcohol and folic acid metabolism antagonist (trimethoprim, seizure medications)

26
Q

B12 Deficiency Characteristics

A
  • B12 from animal products
    1. pernicious anemia most common (lack of intrinsic factor)
    2. strict vegan diet
    3. Crohns disease
    4. Metformin use
27
Q

B12 Clinical Features

A
  1. smooth tongue
  2. glossitis
  3. cheilosis
  4. neuro findings: stocking-glove paresthesias (loss of position, fine touch, vibration), balance problems, dementia
28
Q

B12 DX

A
  1. hypersegmented neutrophils on smear
  2. low B12
  3. shilling test (rarely done)
  4. Anti-intrinsic antibodies
  5. ELEVATED MMA and homocysteine
29
Q

B12 Treatment

A
  1. lifelong B12 supplementation IM for pernicious anemia

2. neuro signs/sxs are reversible if treated within 6 months

30
Q

Polycythemia vera Characteristics

A
  1. Slowly progressive bone marrow disorder characterized by:
    - INCREASED RBC
    - JAK2 mutation
  2. Increased RBC= hyperviscosity (decreased cerebral flow and hypercoagability)
  3. secondary causes: hypoxia, cigarette smoking, living @high altitudes, renal tumors
  4. morbidity/mortality from thrombosis
  5. may convert to CML/AML
31
Q

Polycythemia vera Clinical Features

A
  1. headache, dizziness, fullness in head/face, weakness, fatigue, tinnitus, blurred vision, burning, pain, redness of extremities
  2. pruritis after bathing
  3. epitaxis
  4. plethora, hypertension, enlarged retinal arteries
  5. splenomegaly
32
Q

Polycythemia vera Dx

A
  1. High hemocrit
  2. thrombocytosis, leukocytosis
  3. hyperuricemia
33
Q

Polycythemia vera Tx

A
  1. GOLD STANDARD: phlebotomy
  2. myelosuppressive therapy
  3. hydroxyurea
  4. low-dose ASA to reduce thrombosis risk

Heme/Onc (3 decks)

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